Prune-Belly syndrome

Abstract

Prune-belly syndrome is characterised by a constellation of findings including urinary tract defects, abdominal muscle deficiency and crypto-orchidism. In addition a variety of musculoskeletal and visceral malformations have been reported. We present a case of prune belly syndrome in a male infant that survived 6 days. Clincally the abdomen was distended and wrinkly, with a hypogastric tumour, urinary emission through a vesico-cutaneous fistula and absent testes in the scrotum. Necropsy showed muscle in the abdominal wall, atresia of the vesicouretral junction, vesicomegaly, cystic dilatation of the ureters, cystic and dysplastic changes in the kidneys, bilateral crypto-orchidism, hypoplastic lungs. mobile caecum, bilateral equinovarus and dysmorphic facies with low implantation of the ears. Histologically curious koilocytic changes were observed in the glans. Death was the result of renal and respiratory failure.